Excision of pheochromocytoma

Pheochromocytoma is a rare neuroendocrine disease of the adrenal gland where catecholamine-secreting tumors cause hypertension. Pheochromocytoma is present in 0.05% - 0.2% of hypertensive individuals, the incidence of the disease presents equally between men and women with a distribution across age groups but peaks in between 40 and 50 years of age. The classic presentation of the disease is a triad of symptoms including headache, palpitations, diaphoresis with a documented clinical sign of hypertension (present in 90% of patients with pheochromocytoma). However, patients can often present with less definitive symptoms such as tremor, anxiety, flushing, weight loss, and hyperglycemia. While the majority of pheochromocytoma emerges from adrenal tumors, roughly 15-20% can be extra-adrenal in etiology.

During the early part of the 20th century, the perioperative mortality of this disease ranged between 26-50%. As surgery is curative in about 90% of presenting cases, the mortality has decreased to roughly 1% in specialized centers. Surgical resection can be approached in a variety of ways: (1) open laparotomy; (2) laparoscopic transabdominal; and (3) laparoscopic retroperitoneal. Each approach has different indications, advantages and disadvantages, as well as unique line and monitoring choices. The largest North American series published about pheochromocytoma excision described 108 cases, where 90% were conducted laparoscopically, and the perioperative morbidity rate was 13% without a single mortality.

Excision of pheochromocytoma

Anesthesia type	General
Airway	ETT
Lines and access	Arterial line Additional large-bore PIVs +/- Central venous catheter +/- Pulmonary Artery Catheter +/- Epidural catheter (dependant upon approach)
Monitors	Stanford Monitors Invasive blood pressure monitor +/- Central venous catheter +/- Pulmonary Artery Catheter
Primary anesthetic considerations
Preoperative	Preoperative alpha-blockade
Intraoperative	Rapid episodes of extreme hypertension Severe hypotension after adrenal vein ligation Cardiovascular collapse Hyperglycemia Hypovolemia
Postoperative	Residual hypertension Prolonged hypotension (requiring vasopressors) Hyperglycemia/Hypoglycemia
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Preoperative management

Patient evaluation

System	Considerations
Neurologic	Investigate headaches and fatigue
Cardiovascular	Evaluate history of chest pain, palpitations, arrythmia and signs of heart failure Patients may require EKG or echocardiography Patients may present with catecholamine-induced, Takotsubo, or dilated cardiomyopathy
Respiratory	Classify obstructive or restrictive lung disease
Gastrointestinal
Hematologic
Renal	Evaluate electrolyte disturbances Evaluate fluid status as patients are often hypovolemic from catecholamine excess
Endocrine	Patients may be functionally hyperglycemic due to excessive catacholamine release
Other

Labs and studies

• +/ - electrocardiogram to investigate palpitations, arrhythmia, cardiac ischemia, bundle branch block, or left ventricular hypertrophy
• +/- echocardiogram to assess signs of heart failure, Takotsubo cardiomyopathy, or to diagnose cardiac paragangliomas
• Capillary glucose to test temporary insulin resistance

Operating room setup

Infusion manifold with high rate carrier

Vasopressor infusion (typically non-direct sympathomimetics such as vasopressin)

Direct vasodilator infusion

+/- insulin infusion to treat hyperglycemia

Procedure variants

	Open Laparotomy	Transabdominal Laparoscopic	Retroperitoneal Laparoscopic
Unique considerations			High Insufflation pressures (20-30mmHg)
Position	Supine	Lateral	Prone
Surgical time	4-6 hrs	3-5 hrs	1.5 hrs
EBL			60-100mL
Postoperative disposition	PACU or ICU	PACU or ICU	PACU
Pain management	Epidural	Oral and IV pain medications	Oral pain medications
Potential complications			Subcutaneous emphysema